Macular Telangiectasia type 2: multimodal assessment of retinal function and microstructure.
Müller cells
macular telangiectasia type 2
microperimetry
multifocal electroretinogram
retina
Journal
Acta ophthalmologica
ISSN: 1755-3768
Titre abrégé: Acta Ophthalmol
Pays: England
ID NLM: 101468102
Informations de publication
Date de publication:
Sep 2022
Sep 2022
Historique:
revised:
22
10
2021
received:
28
05
2021
accepted:
19
11
2021
pubmed:
3
12
2021
medline:
12
8
2022
entrez:
2
12
2021
Statut:
ppublish
Résumé
To assess the impact of neurodegenerative morphologic alterations due to macular telangiectasia type 2 (MacTel) on microperimetry (MP) and multifocal electroretinography (mfERG). Thirty-five eyes of 18 patients with MacTel were examined using spectral domain optical coherence tomography (SD-OCT), fundus autofluorescence (FAF), mfERG and MP. Software was used to match SD-OCT B-scans with the corresponding retinal sensitivity map and multifocal electroretinograms (mfERGs), thus enabling direct structure/function correlation. Loss of the ellipsoid zone (EZ) had the strongest negative association with retinal sensitivity (16.77 dB versus 4.58 dB, adj. p < 0.001) of all parameters examined, and a limited negative effect on mfERGs (0.32 SD versus -1.97 SD adj. p = 0.121). Ellipsoid zone (EZ) irregularity was associated with reduced MP values but preserved mfERGs. There was a significant association between areas of inner retinal hyporeflectivity and loss of MP sensitivity (adj. p < 0.001) but the reduction in sensitivity was less than in locations with EZ loss. Areas of mfERG abnormality showed similar sensitivity loss with either inner retinal hyporeflectivity or EZ loss (adj. p = 0.063). In areas with EZ loss alone, preservation of the external limiting membrane (ELM) was associated with higher MP values than in areas with additional ELM loss; the integrity of the ELM alone was not associated with changes either in MP or mfERG. Increased FAF was observed in 51% of eyes, mixed/reduced FAF in 40%, and no abnormality was detected in 9% of eyes. The data suggest both MP and mfERG to be useful non-invasive modalities for detecting localised macular dysfunction in MacTel. The findings suggest a different sensitivity of the two modalities to inner and outer retinal changes in macular function and are therefore complementary.
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
e1240-e1252Informations de copyright
© 2021 The Authors. Acta Ophthalmologica published by John Wiley & Sons Ltd on behalf of Acta Ophthalmologica Scandinavica Foundation.
Références
Balaskas K, Leung I, Sallo FB, Clemons TE, Bird AC & Peto T (2014): Associations between autofluorescence abnormalities and visual acuity in idiopathic macular telangiectasia type 2: MacTel project report number 5. Retina (Philadelphia, Pa) 34: 1630-1636.
Barthelmes D, Gillies MC, Fleischhauer JC & Sutter FK (2007): A case of idiopathic perifoveal Telangiectasia preceded by features of cone dystrophy. Eye (London, England) 21: 1534-1535.
Bringmann A, Iandiev I, Pannicke T, Wurm A, Hollborn M, Wiedemann P, Osborne NN & Reichenbach A (2009): Cellular signaling and factors involved in Müller cell gliosis: neuroprotective and detrimental effects. Prog Retin Eye Res 28: 423-451.
Charbel Issa P, Gillies MC, Chew EY, Bird AC, Heeren TF, Peto T, Holz FG & Scholl HP (2013): Macular telangiectasia type 2. Prog Retin Eye Res 34: 49-77.
Chew EY (2012): How prevalent is macular telangiectasia type 2? Ophthalmic Epidemiol 19: 183-184.
Chew EY, Clemons TE, Jaffe GJ et al. (2019): Effect of ciliary neurotrophic factor on retinal neurodegeneration in patients with macular telangiectasia type 2: a randomized clinical trial. Ophthalmology 126: 540-549.
Clemons TE, Gillies MC, Chew EY et al. (2013): Medical characteristics of patients with macular telangiectasia type 2 (MacTel Type 2) MacTel project report no. 3. Ophthalmic Epidemiol 20: 109-113.
Coscas G, Coscas F, Zucchiatti I, Bandello F, Soubrane G & SouIed E (2013): SD-OCT stages of progression of type 2 macular telangiectasia in a patient followed for 3 years. Eur J Ophthalmol 23: 917-921.
Finger RP, Charbel Issa P, Fimmers R, Holz FG, Rubin GS & Scholl HP (2009): Reading performance is reduced by parafoveal scotomas in patients with macular telangiectasia type 2. Invest Ophthalmol vis Sci 50: 1366-1370.
Gantner ML, Eade K, Wallace M et al. (2019): Serine and lipid metabolism in macular disease and peripheral neuropathy. N Engl J Med 381: 1422-1433.
Gass JD & Blodi BA (1993): Idiopathic juxtafoveolar retinal telangiectasis. Update of classification and follow-up study. Ophthalmology 100: 1536-1546.
Gass JD & Oyakawa RT. (1982): Idiopathic juxtafoveolar retinal telangiectasis. Arch Ophthalmol (Chicago, Ill.: 1960) 100: 769-780.
Gillies MC, Mehta H & Bird AC (2015): Macular Telangiectasia type 2 without clinically detectable vasculopathy. JAMA Ophthalmol 133: 951-954.
Goel N, Kumari A, Kumar S & Mehta A (2020): Multifocal electroretinography in patients with macular telangiectasia type 2. Documenta ophthalmologica. Adv Ophthalmol 141: 15-21.
Heeren TF, Clemons T, Scholl HP, Bird AC, Holz FG & Charbel Issa P (2015): Progression of vision loss in macular Telangiectasia type 2. Invest Ophthalmol vis Sci 56: 3905-3912.
Heeren TFC, Kitka D, Florea D et al. (2018): Longitudinal correlation of ellipsoid zone loss and functional loss in macular Telangiectasia type 2. Retina 38(Suppl 1): S20-S26.
Heeren TFC, Tzaridis S, Bonelli R et al. (2019): Dark-adapted two-color fundus-controlled perimetry in macular Telangiectasia type 2. Invest Ophthalmol vis Sci 60: 1760-1767.
Hood DC, Bach M, Brigell M et al. (2012): ISCEV standard for clinical multifocal electroretinography (mfERG) Documenta ophthalmologica. Adv Ophthalmol 124: 1-13.
Hood DC, Seiple W, Holopigian K & Greenstein V (1997): A comparison of the components of the multifocal and full-field ERGs. Vis Neurosci 14: 533-544.
Kihara Y, Heeren TFC, Lee CS et al. (2019): Estimating retinal sensitivity using optical coherence tomography with deep-learning algorithms in macular Telangiectasia type 2. JAMA Netw Open 2: e188029.
Ledolter AA, Holder GE, Ristl R, Schmidt-Erfurth U & Ritter M (2018): Electrophysiological findings show generalised post-photoreceptoral deficiency in macular telangiectasia type 2. Br J Ophthalmol 102: 114-119.
Mancl LA & DeRouen TA (2001): A covariance estimator for GEE with improved small-sample properties. Biometrics 57: 126-134.
Narayanan R, Dave V, Rani PK, Chhablani J, Rao HB, Pappuru RR & Jalali S (2013): Multifocal electroretinography in type 2 idiopathic macular telangiectasia. Graefes Arch Clin Exp Ophthalmol 251: 1311-1318.
Oh JH, Oh J, Togloom A, Kim SW & Huh K (2014): Characteristics of cystoid spaces in type 2 idiopathic macular telangiectasia on spectral domain optical coherence tomography images. Retina (Philadelphia, PA) 34: 1123-1131.
Okada M, Robson AG, Egan CA, Sallo FB, Esposti SD, Heeren TFC, Fruttiger M & Holder GE (2018): Electrophysiological characterization of macular Telangiectasia type 2 and structure-function correlation. Retina 38(Suppl 1): S33-S42.
Pauleikhoff L, Heeren TFC, Gliem M et al. (2021): Fundus autofluorescence imaging in macular Telangiectasia type 2: MacTel study report number 9. Am J Ophthalmol 228: 27-34.
Powner MB, Gillies MC, Tretiach M, Scott A, Guymer RH, Hageman GS & Fruttiger M (2010): Perifoveal müller cell depletion in a case of macular telangiectasia type 2. Ophthalmology 117: 2407-2416.
Powner MB, Gillies MC, Zhu M, Vevis K, Hunyor AP & Fruttiger M (2013): Loss of Müller's cells and photoreceptors in macular telangiectasia type 2. Ophthalmology 120: 2344-2352.
Ristl R, Hothorn L, Ritz C & Posch M (2020): Simultaneous inference for multiple marginal generalized estimating equation models. Stat Methods Med Res 29: 1746-1762.
Sallo FB, Leung I, Clemons TE, Peto T, Chew EY, Pauleikhoff D, Bird AC. Correlation of structural and functional outcome measures in a phase one trial of ciliary neurotrophic factor in type 2 idiopathic macular Telangiectasia. Retina (Philadelphia, PA.) 38: S27-S32.
Sallo FB, Peto T, Egan C et al. (2012): The IS/OS junction layer in the natural history of type 2 idiopathic macular telangiectasia. Invest Ophthalmol vis Sci 53: 7889-7895.
Vujosevic S, Heeren TFC, Florea D, Leung I, Pauleikhoff D, Sallo F, Bird A & Peto T (2018): SCOTOMA CHARACTERISTICS IN MACULAR TELANGIECTASIA TYPE 2: MacTel project report No. 7-The MacTel Research Group. Retina (Philadelphia, PA.) 38(Suppl 1): S14-S19.
Wang Q, Tuten WS, Lujan BJ, Holland J, Bernstein PS, Schwartz SD, Duncan JL & Roorda A (2015): Adaptive optics microperimetry and OCT images show preserved function and recovery of cone visibility in macular telangiectasia type 2 retinal lesions. Invest Ophthalmol vis Sci 56: 778-786.
Wilkerson JL, Stiles MA, Gurley JM, Grambergs RC, Gu X, Elliott MH, Proia RL & Mandal NA (2019): Sphingosine kinase-1 Is essential for maintaining external/outer limiting membrane and associated adherens junctions in the aging retina. Mol Neurobiol 56: 7188-7207.
Wong WT, Forooghian F, Majumdar Z, Bonner RF, Cunningham D & Chew EY (2009): Fundus autofluorescence in type 2 idiopathic macular telangiectasia: correlation with optical coherence tomography and microperimetry. Am J Ophthalmol 148: 573-583.
Yannuzzi LA, Bardal AM, Freund KB, Chen KJ, Eandi CM & Blodi B. (2012): Idiopathic macular Telangiectasia. 2006. Retina (Philadelphia, Pa.) 32(Suppl 1): 450-460.